716762

Hindawi Publishing CorporationCase Reports in Obstetrics and GynecologyVolume 2013, Article ID 716762, 3 pageshttp://dx.doi.org/10.1155/2013/716762

Case ReportAneurysm of the Vein of Galen Diagnosed with MRI

Themistoklis Dagklis, Chrysoula Margioula-Siarkou, Stamatios Petousis,Theodoros Xenidis, Aggelos Sapidis, Ioannis Kalogiannidis, and David Rousso

3rdDepartment of Obstetrics andGynaecology, Aristotle University ofThessaloniki, Konstantinoupoleos 49, 54642Thessaloniki, Greece

Correspondence should be addressed to Stamatios Petousis; [email protected]

Received 23 December 2012; Accepted 21 January 2013

Academic Editors: E. Cosmi, K. Nasu, and K. Takeuchi

Copyright 2013 Themistoklis Dagklis et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

We describe the case of a neonate with aneurysmof vein of Galen that was diagnosed prenatally in the 33rd gestational week byMRI.A 27-year-old woman, gravida 2, para 2, was admitted to our department at 33 weeks of gestation with suspected fetal hydrocephaly.Ultrasound examination after admission demonstrated an anechoic, supratentorial, and median mass with regular borders, raisingthe possible diagnosis of an aneurysm of the vein of Galen. MRI confirmed the presence of an aneurysm of the vein of Galen. Anelective caesarean section was performed at 33 weeks of gestation.The newborn was admitted to the Neonatal Intensive Care Unit.Despite the full respiratory and medical support given, the sustainment of cardiac failure resulted in neonatal death just one dayfollowing its admission.

1. Introduction

The aneurysm of the vein of Galen represents a rareintracranial arteriovenous malformation, accounting for 1%of all fetal arteriovenous vascular abnormalities [1]. Mainlycorrelated with the persistence of the embryonic medianprosencephalic vein of Markowski [2], the dilatation ofvein of Galen may have a severely detrimental impact onfetal or neonatal heart function, resulting in increased ratesof neonatal morbidity and mortality [3]. As the vein ofGalen develops multiple connections with cerebral arteries,an increased cardiac preload is produced, leading to severeheart failure as early as in fetal life [4]. The prognosis incases of vein of Galen aneurysmal malformations (VGAM)is often poor, with quick development of multisystem organfailure despite vigorous management [5]. Recent advances insupportive medical care and innovation such as embolismhave helped improve the outcome in cases with VGAM [3].Early detection is crucial in order to proceed to effectivetherapeutic management.

We describe the case of a neonate with aneurysm of veinof Galen that was diagnosed in the 33rd gestational week byMRI.

2. Case Report

A 27-year-old woman, gravida 2, para 2, was admitted toour department at 33 weeks of gestation with suspected fetalhydrocephaly during her routine third trimester ultrasound.A fetal medicine specialist ultrasound examination at 22weeks of gestation had not revealed any fetal abnormalities.Ultrasound examination after admission demonstrated ananechoic, supratentorial, and median mass with regular bor-ders, raising the possible diagnosis of an aneurysm of the veinof Galen (Figure 1). Fetal hydrops along with cardiomegalywere also observed. Doppler examination indicated turbulentblood flow of arterial and venous vessels. Blood examinationswere performed, and a blood sample was sent to excludeinfection by Parvo-19.

As soon as ultrasound examination raised the possibilityof a VGAM, an MRI was performed in order to thoroughlyexamine the fetal brain, heart, and chest. MRI confirmedthe presence of an aneurysm of the vein of Galen (Figure 2).An elective caesarean section was performed at 33 weeks ofgestation, and a male neonate weighing 2970 gm was born.The newborn was admitted to the Neonatal Intensive CareUnit. Full respiratory and medical support was given but the

2 Case Reports in Obstetrics and Gynecology

Figure 1: Ultrasound imaging of our cases aneurysm of vein ofGalen.

Figure 2: Fetal MRI imaging of aneurysm of vein of Galen.

sustainment of cardiac failure resulted in neonatal death justone day following its admission.

3. Discussion

The management of the aneurysm of the vein of Galenis a great challenge, especially in case cardiac function iscompromised at the time of diagnosis. Embolism is reportedto be the optimal therapeutic strategy; however, it should beperformed after stabilization of the neonate and preferablyafter the 5th or 6th month of life, with the exception oflife-threatening conditions [6, 7]. Embolism is probably con-traindicated when cardiomegaly or brain injury during laboris present [8]. Therefore, regarding complicated neonateswith early development of severe heart dysfunction as in ourcase, vigorous respiratory and heart support in the NeonatalIntensive Care Unit is the first priority, keeping in mind thatthe prognosis is extremely poor [1].

Prenatal findings indicative of heart dysfunction areassociated with a poor prognosis for the neonate. Sepulvedaet al. [9], in a review of 23 cases with a diagnosis of ananeurysm of the vein of Galen, reported that in 10 of 13 caseswith prenatally diagnosed cardiomegaly there was a neonatalor infant death. No information about long-term outcomeswas available for the survivors. Frawley et al. [3] suggest thataggressive medical treatment and early neurointervention

may contribute to improved clinical outcome even in cases inwhich severe heart dysfunction is already present at delivery.

Early detection of VGAM is crucial for optimal therapeu-tic approach. The diagnostic accuracy of ultrasound Dopplerexamination has already been advocated [10]. Rios et al.supported the usefulness of 3-diamensional Doppler assess-ment in the diagnostic approach of AVG as power Dopplerhas the capacity to obtain a signal in vessels with low-flowvelocity and offers the possibility of a high-accuracy imagingreconstruction of vessels architecture [11]. The contributionof MRI in the diagnosis of VGAM is advocated by Rodeschet al. [6] as it can assist in the early detection of VGAMor in the postnatal therapeutic management, offering thepossibility of deciding on the optimal time for interventionthroughout consecutive screening. In our case, the MRI washelpful in confirming the diagnosis of VGAM that was firstsuspected on ultrasound.

In conclusion, we reported the case of vein of Galenwith coexisting severe heart dysfunction diagnosed prena-tally by ultrasound and MRI. Early detection of VGAM isimportant to maximize the chances of a favorable outcomein complicated cases with early development of severe heartdysfunction.

References

[1] S. Santo, L. Pinto, N. Clode et al., Prenatal ultrasonographicdiagnosis of vein of Galen aneurysmsreport of two cases,Journal of Maternal-Fetal and Neonatal Medicine, vol. 21, no. 3,pp. 209211, 2008.

[2] C. A. Raybaud, C.M. Strother, and J. K.Hald, Aneurysms of thevein of Galen: embryonic considerations and anatomical fea-tures relating to the pathogenesis of the malformation, Neuro-radiology, vol. 31, no. 2, pp. 109128, 1989.

[3] G. P. Frawley, P. A. Dargaville, P. J. Mitchell, B. M. Tress, andP. Loughnan, Clinical course and medical management ofneonates with severe cardiac failure related to vein of Galenmalformation, Archives of Disease in Childhood, vol. 87, no. 2,pp. F144F149, 2002.

[4] S. B. Vijayaraghavan, S. Vijay,M. R. Kala, andD.Neha, Prenataldiagnosis of thrombosed aneurysm of vein of Galen, Ultra-sound in Obstetrics and Gynecology, vol. 27, no. 1, pp. 8183,2006.

[5] Y. Yamashita, T. Abe, N. Ohara et al., Successful treatment ofneonatal aneurysmal dilatation of the vein of Galen: the role ofprenatal diagnosis and trans-arterial embolization, Neuroradi-ology, vol. 34, no. 5, pp. 457459, 1992.

[6] G. Rodesch, F. Hui, H. Alvarez, A. Tanaka, and P. Lasjaunias,Prognosis of antenatally diagnosed vein of Galen aneurysmalmalformations,Childs Nervous System, vol. 10, no. 2, pp. 7983,1994.

[7] C. Garel, M. Azarian, P. Lasjaunias, and D. Luton, Pial arte-riovenous fistulas: dilemmas in prenatal diagnosis, counselingand postnatal treatment. Report of three cases, Ultrasound inObstetrics and Gynecology, vol. 26, no. 3, pp. 293296, 2005.

[8] B. V. Jones,W. S. Ball, T. A. Tomsick, J. Millard, and K. R. Crone,Vein of Galen aneurysmal malformation: diagnosis and treat-ment of 13 children with extended clinical follow-up,AmericanJournal of Neuroradiology, vol. 23, no. 10, pp. 17171724, 2002.

Case Reports in Obstetrics and Gynecology 3

[9] W. Sepulveda, C. C. Platt, and N.M. Fisk, Prenatal diagnosis ofcerebral arteriovenousmalformation using colorDoppler ultra-sonography: case report and review of the literature, Ultra-sound in Obstetrics & Gynecology, vol. 6, no. 4, pp. 282286,1995.

[10] R. Mai, A. Rempen, and P. Kristen, Prenatal diagnosis andprognosis of a vein of Galen aneurysm assessed by pulsed andcolor Doppler sonography,Ultrasound in Obstetrics &Gynecol-ogy, vol. 7, no. 3, pp. 228230, 1996.

[11] L. T. M. Rios, E. A. Junior, L. M. M. Nardozza, A. F. Moron, andM. D. G. Martins, Prenatal diagnosis of an aneurysm of thevein of galen by three-dimensional power and color Dopplerultrasonography, Clinical Medicine Insights: Case Reports, vol.5, pp. 7780, 2012.

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